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What Is Chronic Thromboembolic Pulmonary Hypertension

What Every Physician Needs To Know:

What is Chronic Thromboembolic Pulmonary Hypertension Part 1: Diagnosis | AHN CVI

Prospective observational studies suggest that the cumulative incidence of chronic thromboembolic pulmonary hypertension is in the range of 0.57-3.8 percent following an episode of acute pulmonary embolism. Survival without intervention is poor and, similar to other forms of pulmonary hypertension, is proportional to the degree of pulmonary hypertension and right ventricular dysfunction at the time of diagnosis.

What Laboratory Studies Should You Order To Help Make The Diagnosis And How Should You Interpret The Results

Transthoracic echocardiography â Echocardiography commonly provides the initial objective evidence that pulmonary hypertension is present and that it is not the result of primary left ventricular dysfunction, valvular disease, or, when performed with a contrast study, an intracardiac shunt.

Chest radiography â Pleural abnormalities or regions of avascularity on a chest radiograph can suggest a chronic thromboembolic basis for the pulmonary hypertension.

Pulmonary function testing â Approximately 20 percent of patients will demonstrate a mild restrictive ventilatory defect that appears to be related to parenchymal scarring associated with acute embolism. A reduced diffusion capacity for carbon monoxide is common and appears to be predominantly related to a reduction in pulmonary membrane diffusion capacity.

Arterial blood gas â Some degree of hypoxemia is common and is related to a moderate ventilation-perfusion inequality and a depressed mixed venous saturation resulting from limitation in cardiac output.

Treating Cteph With Surgery

During the PTE procedure, first, youre put on a heart-lung bypass machine to provide blood flow to your other organs. Then, your heart is stopped while the surgeon removes all clots from the arteries in your lung.

The good news is that PTE surgery results in significant improvement in most patients and a return to normal PAP. Its important that you follow up with your provider closely after this surgery so that he or she can monitor you for increases in PAP and determine the best treatment if you have PH following surgery.

If you cant have PTE, you may be a candidate for a newer treatment called balloon pulmonary angioplasty . In this procedure, the doctor uses a tiny balloon that he or she inflates inside the pulmonary artery to widen it. This treatment can lower PAP and help reduce strain on the right side of your heart.

In addition, medications are available if you cant have surgery or still have some PH after PTE.

  • Make sure your provider sends you to a medical center that has doctors experienced in PTE surgery
  • You will be in the hospital for about 2 weeks
  • Your shortness of breath should be better right after surgery
  • You may have pain from the cut and stitches in your breastbone
  • It takes about 2 months for the bone to heal
  • You should not drive, lift anything heavier than 10 pounds, or get the wound wet

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Ready For An Appointment

Before your first appointment, the CTEPH team will review all your medical records and tests. Most of our patients who meet the initial criteria for treatment PTE surgery or balloon angioplasty are able to have a virtual video appointment before their initial consultation. If you’re a candidate for PTE surgery or balloon angioplasty, you will come in for a safe in-person visit with your CTEPH team to receive additional testing before surgery.

If you’re experiencing signs or symptoms of CTEPH, schedule an appointment or call 800-TEMPLE-MED today.

About Chronic Thromboembolic Pulmonary Hypertension

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  • Chronic thromboembolic pulmonary hypertension is a rare disease.
  • The leading most common symptom of CTEPH is shortness of breath which can be difficult to diagnose since its a common symptom of many other conditions.
  • Many patients with CTEPH can be cured with surgery. For patients who cant have surgery, there are medications and other treatments that can help reduce symptoms and provide some relief.

Chronic thromboembolic pulmonary hypertension is a rare and progressive form of pulmonary hypertension . CTEPH is caused by blood clots that dont dissolve in the lungs. These clots cause scar-like tissue that clogs up or narrows the small blood vessels in the lungs.

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Rotational Angiography And Cone Beam Ct

This combination utilises flat-panel technology to provide intraprocedural images using either three-dimensional rotational angiography or cone-beam CT . The CT images have high spatial resolution, but lower contrast resolution, increasing susceptibility to beam-hardening artefacts and noise. Image merging produces a three-dimensional roadmap that can be used in conjunction with real-time fluoroscopy, and provides the possibility of live monitoring from any angle and magnification. This can provide invaluable guidance for complex pulmonary vascular interventions .

What Is The Prognosis For Patients Managed In The Recommended Ways

The short-term and long-term hemodynamic outcomes are favorable following PTE. The pulmonary artery pressure and pulmonary vascular resistance are dramatically reduced and at times normalized. In published series, the mean reduction in pulmonary vascular resistance has approximated 70 percent, and a pulmonary vascular resistance in the range of 200 to 350 dyne·s·cm-5 can be achieved. A corresponding improvement in right ventricular function determined by echocardiography, gas exchange, exercise capacity, and quality of life has also been reported.

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How Is Chronic Thromboembolic Pulmonary Hypertension Diagnosed

If your healthcare provider suspects CTEPH, they will examine you and then may order one or more of the following tests:

  • Lung ventilation-perfusion scan : This type of test uses X-ray scanning equipment to take pictures of what is happening in your lungs. The ventilation portion calls for you to breathe in a little bit of a radioisotope gas-oxygen mixture. The perfusion portion calls for an injection of the radioisotope into your veins. The special X-rays can then determine if there is blockage due to a clot and how well blood and air moves in your lungs. A chest X-ray is usually done before or after a lung V/Q scan.
  • Echocardiogram : Sonography uses sound waves to make pictures of your heart and can estimate the pressure in the lung arteries. This painless test is often done early in the diagnosis process.
  • Computed tomography scan: Contrast material is injected into your vein and X-ray pictures are taken. The pictures will show blood clots.
  • Right-heart catheterization: This test, which uses a catheter placed in the neck or groin, measures blood pressure in the right side of the heart and lungs. This is the most accurate test for pulmonary hypertension.
  • Angiography of lung : In tests of these kind, dye is injected into the arteries via a catheter put into a vein in the neck or groin. Pictures are taken. The dye highlights blockages in arteries of the lungs.
  • Pulmonary function tests: These tests measure how well the lungs are working and the extent of damage to tissues.

Patient Selection For Bpa

Chronic thromboembolic pulmonary hypertension (CTEPH) What Patients Should Know

BPA is still reserved for patients with inoperable CTEPH. Because operability assessment is complex and multifactorial, a multidisciplinary team approach consisting of pulmonary vascular medicine specialists, interventional cardiologists, PTE surgical experts, and imaging specialists is recommended for evaluating and identifying appropriate patients with CTEPH for BPA . Surgical inoperability is a contentious issue and highly dependent on local surgical expertise, patient comorbidities, proximal versus distal pulmonary segmental disease, and patient preference . At experienced centers around the world, segmental and subsegmental resection can be performed to great effect, whereas at less experienced centers, resection may be limited to disease located at the level of the main and lobar vessels. Once a patient has been deemed inoperable a secondary assessment is made to determine if the patient is suitable for BPA. Once again, an assessment must be made regarding the degree of observed vascular obstruction, hemodynamic impairment and symptoms. In addition, assessment of the specific lesion types must be made as certain lesion types confer greater procedural risk and lower success rates .

43.2 ± 11.0 to 24.3 ± 6.4 after BPA 22.5 ± 5.4 at follow-up 96.8% at 1 to 2 yrs94.5% at 3 yrs

mPAP = mean pulmonary artery pressure other abbreviations as in and .

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What Diagnostic Procedures Will Be Helpful In Making Or Excluding The Diagnosis Of Chronic Thromboembolic Pulmonary Hypertension

The reference standard for the diagnosis and determination of surgical accessibility remains combined right heart catheterization and conventional pulmonary angiography .

Five angiographic patterns have been described in chronic thromboembolic disease that correlate with findings at the time of surgery. pouch defects pulmonary artery webs or bands intimal irregularities abrupt, often angular narrowing of the major pulmonary arteries and complete obstruction of main, lobar, or segmental vessels at their point of origin .

Figure 3.

Pulmonary angiogram of a patient with chronic thromboembolic pulmonary hypertension that demonstrates complete obstruction of a major pulmonary artery.

Risk Factors For Cteph

Anyone can develop CTEPH, but there are certain factors that put you at a higher risk. If you have blood clots in the lungs, known as acute pulmonary embolisms , you are at a higher risk. Roughly 75 percent of people with CTEPH have had at least one blood clot before. However, you can still develop CTEPH without ever having a PE.

  • Undergoing thyroid hormone replacement therapy

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Management Of Inoperable Cteph

PAH-targeted therapies are widely used off label in the treatment of CTEPH . Retrospective data from a single center suggest that pre-treating patients with PAH-targeted therapy had no impact on surgical outcomes but was associated with a delay in referral for surgical evaluation . Efforts are currently underway to prospectively study the impact of bridging therapy or pre-treating patients with PAH therapies before PTE.

Table 3. Pulmonary Hypertension Medical Therapy Used for the Treatment of CTEPH

Drug Class

Randomized, double blind, placebo-controlled pilot study

Improvement in World Health Organization functional class and pulmonary vascular resistance after 12 weeks

Retrospective cohort study in severe inoperable CTEPH

Improvement in pulmonary vascular resistance, pulmonary artery pressure, and exercise capacity after 3 months

AIR study Improvement in New York Heart Association functional class and 6-min walk distance at 12 weeks

Uncontrolled trial in severe inoperable CTEPH

Improved pulmonary vascular resistance after 20 months, and higher 5-yr survival rate

Relation To Pulmonary Thrombectomies

Chronic Thromboembolic PH Diagnosis and Management Update: Expert Q& A
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PTEs and are both operations that removed thrombus from the lung’s arterial vasculature. Aside from this similarity they differ in many ways.

  • PTEs are done on a nonemergency basis while pulmonary thrombectomies are typically done as an emergency procedure.
  • PTEs typically are done using hypothermia and full circulatory arrest.
  • PTEs are done for chronic pulmonary embolism, thrombectomies for severe acute pulmonary embolism.
  • PTEs are generally considered a very effective treatment, surgical thrombectomies are an area of some controversy and their effectiveness a matter of some debate in the medical community.

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What Imaging Studies Will Be Helpful In Making Or Excluding The Diagnosis Of Chronic Thromboembolic Pulmonary Hypertension

Ventilation-perfusion scanning represents an essential and noninvasive means of differentiating disorders of the peripheral pulmonary vascular bed from those of the central vasculature.

In chronic thromboembolic disease, at least oneâand more commonly severalâsegmental or larger mismatched perfusion defects are present . In disorders of the distal pulmonary vascular bed, perfusion scans are either normal or they exhibit a âmottledâ appearance characterized by subsegmental defects.

Figure 1.

Ventilation perfusion lung scan of a patient with chronic thromboembolic pulmonary hypertension.

The magnitude of the perfusion defects in chronic thromboembolic disease often understates to a considerable extent the degree of pulmonary vascular obstruction determined angiographically or at surgery. The presence of even a single mismatched, segmental ventilation-perfusion scan defect in a patient with pulmonary hypertension should raise concerns regarding a potential thromboembolic basis.

Mismatched segmental or larger defects in patients with pulmonary hypertension may also arise from other processes that result in obstruction of the central pulmonary arteries or veins, such as pulmonary artery sarcoma, large-vessel pulmonary vasculitides, extrinsic vascular compression by mediastinal adenopathy or fibrosis, and pulmonary veno-occlusive disease.

How Does Cteph Differ From Pulmonary Emboli

Lots of people develop blood clots in the lung arteries but usually your body breaks down the clots within 3 months and you return to normal. Patients with PEs will probably be given warfarin or another anticoagulant to prevent any new clots forming. Unfortunately, in a small number of patients who have a PE the blood clots do not break down fully and the patient will develop CTEPH.

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Preparing For The First Visit

Working with a medical team to find a diagnosis can be a long process that will require more than one appointment. Make better health decisions by being prepared for the first visit with each member of the medical team.

Make informed decisions about health care:

  • Prepare a list of questions and concerns before the appointment
  • List the most important questions first, not all questions may be answered in the first visit
  • Ask questions about symptoms, possible diagnoses, tests, and treatment options

For future appointments:

  • Discuss what was not addressed at the last visit
  • Discuss changes in the quality of life for the patient, family, and caregivers
  • Discuss health goals and other issues in the patientâs and familyâs life that may affect the health care decisions

Description Of The Surgery

Chronic Thromboembolic Pulmonary Hypertension

A PTE has significant risk mortality for the operation is typically 5%, but less in centers with high volume and experience. Individuals with favorable hemodynamic risk profiles also demonstrate lower mortality rates . PTEs are risky because of the nature of the procedure. PTEs involve a full , deep and cardioplegia . Actual removal of the embolus is carried out in a .

There are emerging alternative options available that seek to limit neurologic complications resulting from hypothermia and circulatory arrest. Currently these options have not been shown to be superior to the previously described technique. They include use of moderate hypothermia, antegrade cerebral artery perfusion without total circulatory arrest, and negative pressure application to the left ventricle.

It is important to note that acute is a dramatically different procedure. It’s typically performed without hypothermia as the structure of the clot is different, and the emergent nature presents different operative priorities.

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Balloon Pulmonary Artery Angioplasty

Forpatients with inoperable CTEPH or those with residualpulmonary hypertension after surgery, this minimallyinvasiveprocedure might be an option. UPMC is one ofvery few centers in the country offering this treatment.The technique for BPA is similar to a right-heartcatheterization, where a catheter is introduced into thepulmonary artery with a balloon at its tip. The balloon isinflated in the area where the chronic blood clots arelocated, pushing the clots against the wall of the arteryand restoring blood flow. BPA can complement surgeryand/or medical therapy to improve the quality of life ofCTEPH patients.

What Does It Feel Like To Have Cteph

Unfortunately, there is no way for a doctor to tell that you definitely have CTEPH based on symptoms alone. This is because the most common symptoms of CTEPH are also common symptoms of other diseases, such as asthma, chronic obstructive pulmonary disease , heart failure, or acute PE. If you have CTEPH, some things you may experience include:

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Chronic Thromboembolic Pulmonary Hypertension: The Magic Of Pathophysiology

Gérald Simonneau1,2,3,4, Peter Dorfmüller5,6, Christophe Guignabert1,4, Olaf Mercier1,3,4, Marc Humbert1,2,4

1 2 Assistance Publique Hôpitaux de Paris, Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension Referral Centre, Hôpital Bicêtre , 3 Department of Thoracic and Vascular Surgery, Hôpital Marie Lannelongue, Groupe Hospitalier Paris Saint Joseph, Le Plessis-Robinson , Hôpital Marie Lannelongue, Le Plessis Robinson , German Centre for Lung Research , , Germany

Correspondence to:

Keywords: Chronic thromboembolic pulmonary disease chronic thromboembolic pulmonary hypertension pathophysiology microvasculopathy pulmonary hypertension

Submitted Jul 22, 2021. Accepted for publication Aug 26, 2021.

doi: 10.21037/acs-2021-pte-10

Video

Cteph Treatment At Upmc

Chronic Thromboembolic Pulmonary artery Hypertension

UPMC’s Comprehensive Pulmonary Hypertension Program is one of only a few programs nationwide specializing in treatment of CTEPH. Because this disease is so rare and has similar symptoms to other cardiopulmonary conditions, diagnosing and treating this disease requires the kind of multi-disciplinary expertise that is the hallmark of our physicians.

This disease is potentially curable, and UPMC’s experts are well-skilled in providing treatment options that are best suited for each person, even at advanced stages of disease. When properly treated, the survival rates of people with CTEPH are comparable to those without any history of pulmonary hypertension.

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How Can An Acute Pe Lead To Cteph

An acute PE can increase the risk of developing CTEPH, especially in those who:

  • Have experienced more than one PE in their life

  • Have very high blood pressure in their lungs arteries when their PE is discovered

  • Experience high blood pressure in the lungs for at least 6 months after their PE has been treated

Cteph: Why Is It Important For The Cardiologist

Over the past 510 years, there have been major advances in the field of pulmonary hypertension. Underpinning this is an increasing understanding of the cellular, molecular and genetic mechanisms underlying pulmonary arterial hypertension , coupled with an increased range of effective treatment options for patients with this condition. This has created renewed interest among health professionals in general, and a significant increase in the number of referrals of patients with suspected pulmonary hypertension for assessment and treatment.

As a result of this increased profile, the World Health Organization has sponsored a number of expert consensus conferences to clarify the classification and treatment of pulmonary hypertension.3 Within the revised classification, CTEPH is recognised as a separate entity on the basis of its unique aetiology, and the recognition that it is potentially amenable to surgical cure. This mandates CTEPH being specifically considered in the diagnostic algorithm for any patient presenting with pulmonary hypertension.

There have been several recent comprehensive reviews of both CTEPH and pulmonary endarterectomy.4,5,6 The aim of this article will therefore be to highlight recent advances in our understanding of this condition and areas of controversy and difficulty, particularly where there is a direct impact on clinical practice.

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